Epilepsy and Cortical Dysplasias.
1Department of Neurology, University of Pennsylvania Medical Center, 3 West Gates Building, 3400 Spruce Street, Philadelphia, PA 19104, USA. crinop@mail.med.upenn.edu
Current Treatment Options in Neurology
|November 30, 2000
Summary
Focal and diffuse cortical dysplasias (CD) are brain malformations causing difficult-to-treat epilepsy. While anti-epileptic drugs are standard, epilepsy surgery offers curative potential for select patients with these conditions.
Area of Science:
- Neurology
- Neuroscience
- Epileptology
Background:
- Focal and diffuse cortical dysplasias (FCD/DCD) are heterogeneous disorders characterized by abnormal cerebral cortical cytoarchitecture.
- These conditions are frequently associated with epilepsy, often refractory to anti-epileptic drugs (AEDs), even with polytherapy.
Purpose of the Study:
- To review the diverse etiologies, clinical presentations, and therapeutic strategies for focal and diffuse cortical dysplasias.
- To highlight the significant role of epilepsy surgery in managing refractory epilepsy associated with cortical dysplasias.
Main Methods:
- Review of existing literature on cortical dysplasias and associated epilepsy.
- Analysis of therapeutic outcomes, including anti-epileptic drugs and surgical interventions.
Main Results:
- Cortical dysplasias have diverse etiologies, including genetic syndromes and acquired insults.
- Nearly 30% of epilepsy specimens show overt or microscopic cortical dysplasia.
- While standard AEDs are the mainstay, specific AEDs offer limited advantages; ketogenic diet benefits a subset of patients.
Conclusions:
- Epilepsy surgery, including focal resections, lobectomies, or hemispheric procedures, can be curative in appropriately selected patients with FCD/DCD.
- Understanding the specific type and etiology of cortical dysplasia is crucial for optimizing treatment strategies.
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